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显性遗传性感音神经性聋24家系调查报告
引用本文:王幼勤,薛晓红.显性遗传性感音神经性聋24家系调查报告[J].听力学及言语疾病杂志,1998,6(2):78-80.
作者姓名:王幼勤  薛晓红
作者单位:贵州省人民医院听力中心!贵阳550002
摘    要:文中对24个显性遗传性感音神经性聋家系进行了调查.24个家族共有聋人156人,对其中50人作了纯音测听和声导抗测试,对10名幼儿作了听性脑干反应测试.常染色体显性遗传性聋表现为连续3代以上相传,子代两性均有发病,听力测试为双耳感音神经性聋.应劝阻有遗传性聋者间通婚.对迟发型常染色体显性遗传性聋家族中年幼者应注意听力监测,以便早期发现听觉障碍.早期进行干预.

关 键 词:常染色体  显性遗传  隐性遗传  感音神经性聋

Dominant Inheritance Sensorineural Hearing Loss in Twenty - Four Families
Wang Youqin,Xue Xiaohong,Xu Suqin,et al..Dominant Inheritance Sensorineural Hearing Loss in Twenty - Four Families[J].Journal of Audiology and Speech Pathology,1998,6(2):78-80.
Authors:Wang Youqin  Xue Xiaohong  Xu Suqin  
Abstract:There were one hundred and fifty - six sensorineural hearing loss in twenty-four families. Fifty patients of them were tested with pure tone audiometry and acoustic immitance and ten children with ABR test. The autosomal dominant (AD) inheritance was continuous more than three generations. The descendants might be bilateral sensorineural hearing loss. So, it is nesessary to dissuade the patients from marriage. In the meantime, the younger in the family with delay AD deafness should be observed.
Keywords:Autosomal Dominant inheritance Recessive inheritance Sensorineural hearing loss
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